作为系统性红斑狼疮并发症的肠假性梗阻:是时候重新考虑手术的作用了吗?44岁男性患者多学科综合治疗的病例报告

M. Romeo, M. Dallio, R. Pellegrino, F. Lucido, S. Parisi, C. Gambardella, I. Panarese, Anna Russo, L. Docimo, Alessandro Federico
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引用次数: 0

摘要

肠假性梗阻(IPO)是一种未明确的运动障碍疾病,其特征是临床和放射学表现为与器质性疾病无关的肠梗阻。肠假性梗阻可能是自身免疫性结缔组织疾病(包括系统性红斑狼疮)的一种罕见并发症。外科手术被认为在 IPO 患者的治疗中处于边缘地位:本病例研究了外科手术在诊断和治疗 IPO 急性并发症的多学科方法中的作用。 一名受系统性红斑狼疮影响的 44 岁男性因便秘、呕吐和意外体重减轻被送入我科,他曾反复出现非特异性腹部亚梗阻发作。临床检查显示他有嗜酸性粒细胞性胃肠炎(EG)家族史。包括系统性硬化症筛查在内的实验室检查未发现明显异常。腹部X光片显示有多个气液平面;对比增强CT显示扩张的回肠襻周围有液体,回肠襻充满液体,但没有器质性内在/外在梗阻、穿孔或腔外气体的证据。考虑到这些发现,主要怀疑是狭窄型克罗恩病和狭窄并发的 EG。然而,回肠结肠镜检查和组织学分析结果显示,克罗恩病和肠胃炎并无定论。 组织学检查显示,在右半结肠切除术中采集的手术样本中,肠壁浸润了系统性红斑狼疮纤维炎症模式,但没有忽略肠神经丛,因此可以诊断为 IPO。 事实上,该患者最初接受了保守治疗,由包括胃肠病专家、风湿病专家和营养学家在内的多学科团队进行鼻胃减压、免疫抑制治疗和肠外营养。不幸的是,2 周后症状恶化,需要进行右半结肠切除术和回肠造口术以挽救生命。 系统性红斑狼疮患者出现治疗无效的消化道症状时,应怀疑患有 IPO。及早发现并采取针对性的多学科治疗方法对预后至关重要。手术是诊断的关键时刻,也是挽救生命的治疗方法。
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Intestinal pseudo-obstruction as systemic lupus erythematosus complication: is it time to reconsider the role of surgery?: A case report of a tailored multidisciplinary managed 44-year-old male patient
Intestinal pseudo-obstruction (IPO) represents an unclarified dysmotility disorder characterized by clinic-radiological signs of intestinal obstruction not associated with organic conditions. IPO may occur as a rare complication of autoimmune connective tissue diseases, including Systemic Lupus Erythematosus (SLE). Surgery is recognized as having a marginal management position for IPO patients: this case investigates its role, in the context of a multidisciplinary approach, in the diagnosis and treatment of acute complications of IPO. An SLE-affected 44-year-old man with a history of recurring nonspecific abdominal sub-obstructive episodes was admitted to our department for constipation, vomiting, and unintentional weight loss. Clinical examination revealed a family history of eosinophilic gastroenteritis (EG). Laboratory tests, including Systemic Sclerosis screening, presented no significant alterations. Abdominal X-ray highlighted multiple air-fluid levels; contrast-enhanced CT showed fluid surrounding dilated loops, and fluid-filled ileal loops, without evidence of organic intrinsic/ab-extrinsic obstruction, perforation, or extraluminal gas. Considering these findings, stenosing-pattern Crohn disease and strictures-complicated EG were mainly suspected. However, ileum-colonoscopy with the histological analysis resulted in inconclusive for Crohn disease and EG. The histological examination, by showing an SLE fibro-inflammatory pattern, not sparing the enteric plexus, infiltrating the intestinal wall of the surgical samples collected during the right hemicolectomy intervention, allowed the diagnosis of IPO. The patient, indeed, was initially managed conservatively with nasogastric decompression, immunosuppressive therapy, and parenteral nutrition by a multidisciplinary team including gastroenterologists, rheumatologists, and nutritionists. Unfortunately, 2 weeks later, the worsening symptoms required a lifesaving right hemicolectomy followed by ileostomy. IPO should be suspected in SLE patients presenting treated-unresponsive GI symptoms. Its early recognition finalized to start a tailored multidisciplinary approach appears prognostically crucial. Surgery could represent a paramount diagnostic moment and therapeutic life-saving approach.
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